NATIONAL FRAMEWORK FOR NEONATAL HEARING SCREENING

August2013

Why a National Approach to Neonatal Hearing Screening?

National Approach to Neonatal Hearing Screening

Purpose and structure of this document

Introduction

Background

Assessing neonatal hearing screening against the AHMAC Population Based Screening Framework

Aims and Objectives of Neonatal Hearing Screening

National Approach to Neonatal Hearing Screening Framework

National Neonatal Hearing Screening Pathway Guidance

Consistent Standards of Practice

Governance

Collaborative Partnership with Other Key Stakeholders

Agreed National Approach to Data Collection, Management and Data Sharing

Registry function

National Evaluation Strategy

Further consideration

Equitable Access

Possible Risks

Recruitment

Screening

Diagnosis (Confirmation and Investigation of Hearing Loss)

Treatment (Early Intervention and Management)

Family Support

Coordination, Monitoring and Evaluation

Professional Education

Framework

Appendix A

Proposed national performance indicators

Appendix B

Membership of the Neonatal Hearing Screening Working Group

Secretariat

Appendix C

Summary of Assessment of Neonatal Hearing Screening against the National Population Based Screening Framework for suitability as a National Population Based Screening Program (considered by the Screening Subcommittee August 2009)

Appendix D: Screening Pathway Guidance

Appendix E

Risk Factors

Appendix F

Professionals Associated with Hearing Impairment

Appendix G

Information required to Australian Hearing in order for an appropriate hearing aid fitting to occur

Appendix H

Draft Standards Framework

1. Recruitment

2. Screening

3. Parent Support

4. Diagnosis (confirmation of hearing loss)

5. Early Intervention and Management

6. Co-ordination, Monitoring and Evaluation

7. Professional Education

Glossary

Why a National Approach to Neonatal Hearing Screening?

There are over 297,900[1] births in Australia annually. All States and Territories in Australia have universal neonatal hearing screening. It is widely acknowledged that delays in the identification and treatment of permanent childhood hearing impairment may profoundly affect quality of life in terms of language acquisition, social and emotional development, and education and employment prospects. It must be noted that approximately 50% of hearing impairment at birth is without a risk factor.[2]

There is clearly a need for a National Framework for universal neonatal hearing screening and early management of interventions in Australia. A national approach will aim to screen all babies in Australia for potential permanent childhood hearing impairment, and provide access to interventions to minimise the impact of hearing impairment.

National Approach to Neonatal Hearing Screening

According to the international literature, moderate to profound (>40 dB) bilateral permanent childhood hearing impairment (PCHI) occurs in 1.3 per 1,000 babies. Unilateral PCHI of similar severity occurs in 0.6 per 1,000 babies. This suggests that, each year in Australia, approximately 331 children are born with bilateral PCHI, and 174 children are born with unilateral moderate to profound PCHI. This is a total of 551 children each year[3].

Children with slight or mild hearing loss (26-40 dB) have not been included in the treatment and early intervention pathway guidance encompassed within this framework. However, families with children with this level of hearing loss should have access to information about how they can monitor their child’s hearing loss and who they might consult if they have concerns about their child’s ongoing development.

It has been suggested that the prevalence of PCHI increases substantially with age. The consequences of the condition include life-long impairment of language skills and possible delays in social development and academic achievement. The severity of the outcome is influenced by the degree and duration of hearing loss, the age at which the hearing loss first appeared, and the hearing frequencies affected. Developmental delays are particularly apparent for children with severe and profound hearing impairment. Children with hearing impairment are more likely to experience lower self-perceived health status than those without hearing loss. Early management of childhood hearing impairment provides many benefits, including improved communication and language ability, mental health, and future employment prospects[4].

Purpose and structure of this document

The purpose of this document is to provide an overarching framework which outlines the principles of a National Approach to Neonatal Hearing Screening. The framework provides high level guidancefor a progressive implementation of Neonatal Hearing Screening in Australia. Some key ideas are prominent in the document, namely the importance of effective communication with, and education of, parents.

This document includes background information and a brief discussion on the merits of a National Neonatal Hearing Program, followed by the National Neonatal Hearing Screening Framework under which the proposed program will operate. The screening pathway guidance is also outlined including a discussion of the major components from screening to post screening follow-up which are:

  • recruitment of the target population;
  • the progression from screening to diagnosis;
  • early intervention, treatment and management;
  • coordination, monitoring and evaluation of screening and early intervention; and
  • necessary professional education that both practitioners and families will have available.

An in depth list of the objectives, standards expected and performance indicators for each part of the program are provided at the end of the document. The objectives, standards and target performance indicators are intended to provide principle-based standards for screening services and post screening follow-up. These can guide the development of implementation specific protocols, clinical guidelines, key performance indicators or accreditation standards.

The National Framework recognises that the development of neonatal hearing screening has developed separately across jurisdictions with various levels of sophistication. The National Framework has been developed in consultation with jurisdictions with an aim of achieving harmonisation of these efforts. It is intended as a resource for jurisdictions to usewhen developing neonatal hearing screening services.

Introduction

Hearing impairment may be categorised as slight or mild, moderate, severe or profound. The grades of hearing impairment differ across organisations and countries. The World Health Organisation has defined hearing loss (in the better ear) in adults:

  • at 26-40 dB as slight or mild hearing impairment. With this impairment, an individual should be able to hear and repeat words spoken in a normal voice at a distance of one metre[5] in an environment with no background noise. Children with this impairment may experience difficulty in comprehending speech and oral language in normal circumstances. The child’s articulation and language development may be compromised. Speech and language usually develop normally if a child is fitted with hearing aids early[6] and is provided with sustained intervention;
  • at 41-60 dB as moderate impairment. With this impairment, an individual can hear and repeat words spoken in a raised voice at a distance of one metre[7] in the absence of background noise. Speech and language development are generally affected unless a hearing aid and quality early intervention are provided;
  • at 61-80 dB as severe hearing impairment. With this impairment, an individual is able to hear some words when shouted into the better ear[8]. However this is inadequate for access and acquisition of spoken language. Speech and language do not develop spontaneously in a child born with this degree of impairment. Hearing aids amplify many speech sounds and will assist a child to develop speech, but speech quality is likely to be affected[9]; and
  • at 81 dB or greater as profound hearing impairment, including deafness. Individuals with this type of impairment are unable to hear and understand a shouted voice[10]. Learning to speak and understand spoken language is difficult for children born with a profound hearing loss. Many children with profound hearing loss are now fitted with a cochlear implant[11].

The target condition for detection and follow up by the Program is defined as congenital permanent bilateral, unilateral sensory or permanent conductive hearing loss including neural hearing loss (e.g. Auditory Neuropathy Spectrum Disorder) of >40 dB.

There is strong evidence that babies whoare identified with moderate-to-profound hearing loss in the first six months of life, and provided withimmediate and appropriate intervention including amplification and/or cochlear implantation as appropriate, have significantly betteroutcomes than later-identified infants and children in vocabularydevelopment, receptive and expressive language syntax,speech production and social-emotional development.Children enrolled in early intervention within the first yearof life have also been shown to have language development withinthe normal range of development at five years of age[12].

Early intervention is necessary to achieve optimal outcomes for hearing-impaired children. Research shows that family involvement is associated with positive language outcomes, and that parental involvement, particularly school based parental involvement (e.g. participation in individual educational plan meetings and parent meetings) will predict early reading skills. Importantly, maternal communicative skills are even more predictive of language and literacy. Maternal communicative skill is a strong aspect of parental involvement, given that a parent must be highly involved to develop effective mutual communication with a deaf or hearing impaired child[13].

Background

In March 2002, the National Health and Medical Research Council (NHMRC) released a report titled ChildHealth Screening and Surveillance: A Critical Review of the Evidence. The Report found that there was evidence to recommend national neonatal hearing screening, but urged serious consideration of the logistics and quality of the testing system, and the follow up systems for babies who test positive, before the implementation of a national neonatal hearing screening program.

In July 2002, the Australian Health Ministers Conference (AHMC) requested the Medical Service Advisory Committee (MSAC) undertake an assessment on the safety, effectiveness and cost-effectiveness of universal neonatal hearing screening. In 2008, MSAC released the Universal Neonatal Hearing Screening Assessment Report[14], which addressed these issues but did not make recommendations with a view to establishing a national neonatal hearing screening program.

In March 2008, the Screening Subcommittee of the then Australian Population Health Development PrincipalCommittee[15] (APHDPC) agreed to examine the feasibility of a national approach to neonatal hearing screening. It established the Neonatal Hearing Screening Working Group[16] with the following terms of reference:

  1. Assess neonatal hearing screening against the Population Based Screening Framework[17].
  2. Develop minimum national standards for screening services and post screening follow-up with regards to audiology, medical intervention, family counselling, early intervention and education.
  3. Consider and develop screening pathway to improve population coverage for neonatal hearing screening in Australia.
  4. Develop a national quality and reporting framework for consideration by the Screening Subcommittee of the Australian Population Health Development PrincipalCommittee and Australian Health Ministers’ Advisory Council.
  5. Establish an agreed national approach to data collection and management and data sharing.

In July 2009, the Council of Australian Governments (COAG) agreed to a proposal that universal neonatal hearing screening would be available in all states and territories by the end of 2010.

Assessing neonatal hearing screening against the AHMAC Population Based Screening Framework

The Population Based Screening Framework takes into account the World Health Organisation (WHO) principles and elaborates on them in the Australian context. The Framework, developed with input from a wide range of experts in screening, is based on the latest available evidence and informed by experience with existing Australian population screening programs[18]. The Framework has been divided into two parts:

  • the criteria used to assess whether screening should be offered, or a screening program introduced, for diseases or conditions; and
  • the key principles for the implementation and management of screening programs.

The Framework, like all population screening programs, is underpinned by the principles of access and equity. It is intended to provide information and guidance on the key issues to be considered in the development of a population screening program in Australia. An assessment of the national approach to neonatal hearing screening against the Framework’s criteria is at Appendix C.

Aims and Objectives of Neonatal Hearing Screening

The aim of neonatal hearing screening is for all babies to be screened for PCHI, and, if necessary, to have access to appropriate intervention to minimise the impact of their hearing impairment. This will improve the quality of life for children with PCHI in terms of their communication and language skills, subsequent education and employment prospects, and psychological wellbeing.

The objectives of neonatal hearing screening are to:

  • maximise the early detection of PCHI in Australian babies through the use of an approved screening test (see page10), and appropriate follow up medical, and support services;
  • ensure that all Australian families are offered the opportunity to participate in neonatal hearing screening;
  • ensure equitable access to neonatal hearing screening for all Australian babies, irrespective of their geographic, socioeconomic or cultural background;
  • ensure that assessment services provided to babies requiring follow up care and intervention as a result of screening are timely, acceptable and appropriate and are undertaken in accordance with professional standards;
  • ensure families with babies diagnosed with impaired hearing are referred and have the opportunity to, engage with an early intervention service following diagnosis;
  • maximise benefit and minimise harm to the individual; and
  • achieve consistent standards of screening management, co-ordination, quality and safety, service delivery, monitoring and evaluation and accountability andensure that the national approach to neonatal hearing screening is implemented in a manner that is cost effective and will significantly increase quality of life for Australian children with PCHI.

National Approach to Neonatal Hearing Screening Framework

There are six major components that could make up the National Neonatal Hearing Screening Framework (National Framework) as Figure 1 illustrates.

Figure 1: Components of the National Neonatal Hearing Screening Framework

The National Framework focuses on having standardised screening pathway guidance across Australia, supported by evidence based standards of practice. It is supported by a national approach to data collection, management and data sharing through a national data set. The National Framework is a joint initiative between the Australian and state and territory Governments in collaboration and partnership with specialists in the field of paediatric hearing and in consultation with families of children with hearing loss.

National Neonatal Hearing Screening Pathway Guidance

The National Neonatal Hearing Screening Pathway Guidance is evidence-based and includes recruitment, initial screening tests to the point of definitive diagnosis and post screening follow up. A flow-chart is at Appendix D. To highlight the continuum of care, the interaction between screening and early intervention and management services is included.The major components of the screening pathway guidance are:

  • Recruitment - The target population is all babies >34 weeks gestation screened within 24 to 72 hours of birth with an aim to complete screening by four weeks corrected age[19]. Corrected age takes into account the time between premature birth and the actual due date of a full term pregnancy. Calculating corrected age provides a truer reflection of what the baby’s developmental progress should be. Protocols should be in place to ensure that screening can occur up to six months of age for babies not screened within the target time frame.
  • Screening - The screening tools used in Australia to identify babies with possible PCHI are currently the transient evoked otoacoustic emissions (TEOAE) test and the automated auditory brainstem response (AABR). It is important that the screening equipment used is validated for sensitivity and specificity for the targeted condition.
  • Diagnosis (confirmation of hearing loss) - A definitive audiological diagnosis and medical investigation needs to be made following a positive result (often referred to as a refer result) on the screen. Recommendations on the appropriate time between screening and diagnostic assessment, referral for medical evaluation and referral to Australian Hearing are included in the standards.

The intervention and management specific pathway components include:

  • Early intervention, treatment and management - all families of babies with a confirmed hearing impairment, bilateral and unilateral, should be provided with unbiased information on the range of services available, including services provided by Australian Hearing and other early hearing intervention services. All families of babies with a confirmed hearing impairment should be referred and have the opportunity to access Australian Hearing and other early intervention services before three months of age for advice, including amplification fitting services and cochlear implantation candidacy evaluation. These services should be provided by professionals with expertise in hearing loss and should be available from time of diagnosis to school entry. Early intervention and management services include both amplification intervention and other early intervention services provided following diagnosis of a confirmed hearing impairment.

Program supporting and enabling components include:

  • Quality Management Plan – A Quality Management Plan will be developed to assist with the planned implementation of quality initiatives to support the delivery of high quality neonatal hearing screening.
  • Coordination, monitoring and evaluation –Registry function/smaybe developed to assist coordination and monitoring and evaluation.
  • Professional education -Families have access to safe and sensitive services provided by appropriately qualified, skilledand experienced professionals. Consideration will need to be given to the training and certification of professionals carrying out screening tests. All professionals, including support workers, should be trained in hearing issues.

Consistent Standards of Practice

The National Framework includes national guidance on standards of practicethat all states and territories will be able to use to ensure a consistent national approach to neonatal hearing screening. These standardsare supported by an evidence-base to ensure quality outcomes. The development of standards of practice has been a collaborative approach between all stakeholders.